Wednesday, August 24, 2011

Good News & Bad News

It has been quite an interesting few days  in the ALS community!  Being my normal upbeat self, let me share the good news first.  This past Sunday researchers at Northwestern University's Feinberg School of Medicine announced that they have discovered the common cause of all types of ALS.  Here's a link to the full article:

"Dr. Teepu Siddique, a neuroscientist with Northwestern University’s Feinberg School of Medicine whose pioneering work on ALS over more than a quarter century fueled the research team’s work, said the key to the breakthrough is the discovery of an underlying disease process for all types of ALS"

"Ubiquilin2 in spinal and brain system cells is supposed to repair or dispose of other proteins as they become damaged. The researchers discovered a breakdown of this function in ALS patients.
When Ubiquilin2 is unable to remove or repair damaged proteins, the damaged proteins begin to pile up in the cells, eventually blocking normal transmission of brain signals in the spinal cord and brain, leading to paralysis."

While I tend to look at all proclamations like these with a very cautious eye, I am daily being persuaded that this could be the real deal.  With the cause identified, scientists can focus their efforts on finding a treatment and eventually a cure.  With positive reports coming in from two current clinical drug trials, NP001 and Dexpramipexole, and the cause identified, it is safe to say that a fresh wind of hope is blowing through the ALS community.  The FDA approval process can be agonizingly slow, especially given the limited life span PALS face, but whether or not it happens in my lifetime, finding the cure is going to happen.

The bad news is that our community has lost another champion warrior to the beast.  I did not have the privilege to meet Tim LaFollette or his lovely wife Kaylan in person, but his work through the Often Awesome web videos was simply amazing.  Tim had the fore sight to chronicle his journey with ALS starting with diagnosis and the resulting 33 episodes have been described as "Tuesdays with Morrie on steroids".  I have been humbled, inspired, humored, terrified and touched to the core by Tim's story - watch the episode below for a taste.  Tim will be greatly missed, condolences to Kaylan and the Often Awesome Army, we will never give up.

Monday, August 8, 2011

Silly Songs

We recently passed the ten year anniversary of my Dad's passing, and he's been in my thoughts a lot lately.  My sister was talking about some of her favorite Sunday dinners that Dad cooked, and some of the lakefront and backyard bar b ques in the summer that we all enjoyed.  Then my cousin brought up another favorite memory, the silly songs he sang to us when we were kids.

You may recall from an earlier post that my Dad was a musician; he loved to entertain us, or maybe we just bugged him until he gave in.  There may have been more, but the three favorite songs brought smiles and giggles to children in our family for years.  Now with the help of the internet, I'll share them with you.

1) Honkety Hank - not sure Dad knew the whole song, but his sound effects made the tune.  I found out this song was written in 1949 by Pinto Colvig, who was the original voice of Bozo the Clown. Here's some of the lyrics:
Young Honkety Hank, a mechanical boy
Built himself the silliest toy
An automobile that went when he run it
The neighbors all said that he shouldn't o' done it.

The poofiest car that a kid ever built
All painted and patched like an old crazy quilt
'Twas made out of boxes and tin cans and such
That didn't have even a brake or a clutch.

'Twould jiggle and twiggle and wiggle and giggle
And mutter and sputter and flutter
Would cough and sneeze and and wheeze
Down the street with a terrible clutter.

That old hunk-a-junk would make anyone laugh
The motor inside was an old phonograph
The wheels were wobbly and bubblegum tires
And all would every time it backfired.

2) The Unicorn Song - Sung in Dad's best Irish accent, this song by The Irish Rovers was a top 10 hit in1968 and is still popular in Irish pubs today

3) My Mule Charley - this was my personal favorite, sorry couldn't find the lyrics but did find an audio snippet.
My Mule Charley
The rest of the song involves a trip to town to buy a horn, Charley getting into the shop and greats sound effects and a glockenspiel. It ended in a rousing rendition of "Oh Susana!"

So these are the songs that we all have such fond memories of, thanks Dad!  Hope the family enjoys this and maybe shares with the next generation of Connell kids.